The objective of this study was to assess exercise performance in subjects born in Sweden between 1980 and 1995 and undergoing surgery for pulmonary atresia and intact ventricular septum and to identify determinants of exercise performance. Twenty-seven subjects, 16 with biventricular repair and 11 with univentricular palliation, and 28 age- and sex-matched controls completed cardiopulmonary exercise and lung function testing. Peak oxygen uptake was determined using a symptom-limited ramp bicycle exercise protocol. Regression analysis was performed to identify predictors of peak oxygen uptake (V'O(2)), The index group had lower peak V'O(2) (1.4 [median 0.8; range 2.5] l/min) than controls (1.9 [0.7; 3.1]; p < 0.05). Subjects without ventriculocoronary arterial communications (VCAC), corrected to biventricular circulation, had higher peak V'O(2), than the remaining index subjects. Decreased total lung capacity, low minute ventilation, and high physiologic dead space measured at peak exercise were all independent determinants of low peak V'O(2) Exercise capacity is generally decreased in subjects with pulmonary atresia and intact ventricular septum, although there are marked interindividual differences. Good exercise capacity was found in subjects without VCAC who had undergone biventricular repair. Decreased lung function was an unfavourable predictor of exercise capacity.